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右心室双出口。对28例连续患者的临床-放射学-病理学研究。

Double-outlet right ventricle. A clinical-roentgenologic-pathologic study of 28 consecutive patients.

作者信息

Guo D W, Lin M L, Gu Z Q, Cheng T O

出版信息

Chest. 1984 Apr;85(4):526-32. doi: 10.1378/chest.85.4.526.

Abstract

Twenty-eight cases of double-outlet right ventricle (DORV) were found from 1975 to 1980 in the Shanghai Chest Hospital. On plain films, four types of abnormal heart configuration were found: mitral, boot-shaped, atypical, and bizarre. The main angiocardiographic findings were: (1) simultaneous visualization of aorta and pulmonary artery on right ventriculography, both arising from the right ventricle; (2) D- or L-type of aortic transposition in most; (3) the two semilunar valves at approximately the same horizontal level; (4) an infundibulum present below the semilunar valve; (5) ventricular septal defect; and (6) associated pulmonary stenosis and/or other anomalies. An anatomic-radiologic classification was suggested and based chiefly on symbolic representation of segmental combinations including visceral trial situs, type of cardiac loop, relation between the semilunar valves, presence or absence of pulmonary stenosis and whether the ventricular septal defect was subpulmonary or not. The findings substantiated the viewpoint that in DORV there exists on embryologic and pathologic ground transitional changes ranging from those closely resembling tetralogy of Fallot to complete transposition of the great vessels.

摘要

1975年至1980年期间,上海胸科医院共发现28例右心室双出口(DORV)病例。在X线平片上,发现了四种异常心脏形态:二尖瓣型、靴型、非典型和奇异型。心血管造影的主要表现为:(1)右心室造影时主动脉和肺动脉同时显影,均起自右心室;(2)大多数为D型或L型主动脉转位;(3)两个半月瓣大致在同一水平;(4)半月瓣下方有漏斗部;(5)室间隔缺损;(6)合并肺动脉狭窄和/或其他异常。提出了一种解剖-放射学分类方法,主要基于节段组合的符号表示,包括内脏心房位置、心脏环类型、半月瓣之间的关系、有无肺动脉狭窄以及室间隔缺损是否位于肺动脉瓣下。这些发现证实了这样一种观点,即从胚胎学和病理学角度来看,右心室双出口存在从与法洛四联症极为相似到完全性大动脉转位的过渡性变化。

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